Twelve patients with Henoch-Schonlein purpura, aged 6-14 years (mean 1
0.3 years), presenting with rapidly progressive glomerulonephritis (RP
GN) were investigated prospectively. Analysis of the initial clinical
features revealed: oedema (8 patients), hypertension (7 patients), gro
ss haematuria (11 patients), oliguria (5 patients) and a decreased glo
merular filtration rate (GFR) (<40 ml/min per 1.73 m(2), 8 patients).
Renal biopsies were available in 9 patients and revealed focal necroti
sing and a fibroepithelial type of crescentic glomerulonephritis (with
60%-90% crescent formation). The remaining 3 patients fulfilled the c
linical criteria of RPGN. Two patients who were in the acute stage req
uired peritoneal dialysis for a period of 2 weeks. The treatment proto
col in all patients consisted of intravenous pulse methylprednisolone
(3 days), oral cyclophosphamide (2 months), oral dipyridamole (6 month
s) and oral prednisolone (3 months). At the end of triple therapy, GFR
returned to normal in all but 1 patient. During a follow-up period of
9-39 months, 7 patients achieved complete remission, while 4 patients
showed partial remission, 3 of whom had persistent proteinuria and ha
ematuria and 1 microscopic haematuria only. One patient had persistent
nephropathy with decreased GFR and macroscopic haematuria and nephrot
ic-range proteinuria. His renal biopsy, performed 30 months after the
onset of the disease, showed chronic diffuse sclerosing glomerulonephr
itis and intratubular severe IgA deposition. Although our patient grou
p was small, this type of intensive treatment appears to be effective;
further studies are needed.